Acta Ophthalmologica最新文献

Aims/Purpose: Retinal oxygen extraction has been found to be altered in several ocular and systemic diseases, such as diabetic retinopathy, glaucoma or neurodegenerative diseases. However, measurement techniques for the assessment of retinal oxygen extraction are limited because most of them are based on time-consuming and custom-built devices. In the present pilot study, we assessed retinal oxygen extraction during systemic hypoxia with a combination of two commercially available devices: Laser-Speckle Flowgraphy (LSFG) and retinal oximetry.

Methods: Retinal blood flow and retinal oxygen saturation were measured in each vessel around the optic nerve head at the same position. Arterial and venous oxygen content was estimated from the measured retinal oxygen saturation parameters and the difference between these two parameters was calculated (cO₂,_DIFF). By using mean vessel flow rate (MV) for retinal blood flow and cO₂,_DIFF, retinal oxygen extraction was determined. For each healthy study participant, baseline measurement and measurement during inhalation of 88% nitrogen in 12% oxygen were performed.

Results: Ten (10) healthy subjects (4 females, 6 males) with a mean age of 27 ± 3 years were included in this study. During systemic hypoxia, a significant decrease in arterial oxygen content occurred (p = 0.046) while venous oxygen content did not change, leading to a decrease in cO₂,_DIFF. As expected, this was accompanied by a significant increase in MV (p = 0.014). No change in retinal oxygen extraction was observed (46 ± 8 a.u. at baseline vs. 46 ± 5 a.u. during hypoxia, p = 0.997).

Conclusions: Breathing of 88% nitrogen in 12% oxygen caused a significant decrease in retinal arterial oxygen content. This was compensated by an increase in retinal blood flow in order to keep retinal oxygen extraction constant. This was physiologically expected, and these findings are consistent with the literature. Therefore, the presented approach for measurement of retinal oxygen extraction appears to be feasible.

Aims/Purpose: The aim of this study is the present a long-time follow up of patients with LCHAD deficiency – ocular findings and it's evolution.

Methods: The study included a group of 8 patients diagnosed with LCHAD deficiency, confirmed by genotyping as a mutation in HADHA gene c.1528 G > C. Patients were evaluated yearly, since 2013 to 2024. Examination included slit-lamp examination, best corrected vision acuity, sOCT, fundus imaging and visual field – if possible to obtain. Patients were questioned about events of metabolic decompensation that required hospital treatment in the period of time since previous ophthalmological evaluation.

Results: Progressive deterioration of BCVA along with progressive myopia were observed. The loss of vision usually progressed rapidly and was unsymmetrical, thus usually unnoticed by the patient if the fellow eye maintained good vision acuity. The deterioration of the vision acuity was accompanied by presence of central scotoma, progression of myopia, chorioretinal thinning and in some cases forming of staphyloma. Pigment accumulation in the foveal area and in the central area was not associated with the vision deterioration or visual field loss. In 2 cases MNV occurred, followed by macular scarring in one patient. MNV if treated by anti-VEGF injections without a delay responded very well, 2-3 injections were needed to maintain the deasease inactive. Positive scotomas were associated with macular hemorhage. Bad metabolic status recognized by repeated necessity of in ward treatment due to metabolic decompensations was associated with progressive chorioretinopathy and it's complications.

Conclusions: Presence of the metabolic decompensations requires ophthalmological vigilance as it is a main factor for visual deterioration. The chorioretinal thinning is followed by staphyloma and progressive myopia. MNV can be successfully treated in those patients but evaluation should be done monthly afterwards.

Aims/Purpose: To report a case of marginal keratitis that developed after intravitreal bevacizumab (Avastin) injection.

Methods: A 88-year-old woman with Age-related macular degeneration (AMD) and macular edema in both eyes received four intravitreal injection of bevacizumab (Avastin) in left eye and five in right eye. No previous history of herpes.

Results: Two days following the fifth injection of Avastin in the patient's right eye, she experienced sensations akin to having a foreign body, accompanied by redness and a decrease in visual acuity in that eye. Examination with a slit lamp revealed peripheral infiltrates beneath the corneal epithelium, delineated from the limbus by a clear zone. Additionally, there was corneal edema with folds in the endothelium and a mild anterior chamber reaction. No presence of hypopyon was noted. The patient's symptoms showed favorable response to topical steroid and topic and oral antibiotic medications

Conclusions: The intravitreal administration of bevacizumab carries a risk of inducing hypersensitivity reactions like marginal keratitis. To our knowledge, this is the first documented account of marginal keratitis after intravitreal bevacizumab injection.

The aim of this part is to show the difference between normal and abnormal findings in the angle. In addition grading systems and their therapeutic consequences will be discussed.

Congenital disorders and acquired diseases affecting the eye and angle will be shown. The clinical findings will be discussed as well its context of recent advances in glaucoma surgery with microsurgical techniques requiring intraoperative gonioscopy for implants.

Aims/Purpose: It is not yet established whether time of idebenone treatment initiation from symptom onset (T_i) impacts the clinical outcome of patients with Leber hereditary optic neuropathy (LHON). LEROS, a Phase IV, open-label, international, natural-history controlled study (NCT02774005), assessed the long-term efficacy of idebenone in the treatment of LHON.¹ In this post-hoc analysis of LEROS study data, we report visual acuity (VA) endpoints by T_i.

Methods: VA endpoints at last visit were assessed as previously described, in idebenone-treated eyes from the LEROS study with an observation time of 12–24 months (m) and a m.11778G > A mitochondrial DNA mutation.¹ In this post-hoc analysis, eyes were stratified by T_i. No statistical comparisons were performed.

Results: Median [range, n] age at baseline was similar regardless of T_i (0–3m: 32.2y [12.6–49.6, 10]; 3–6m: 36.2y [12.6–62.5, 21]; 6–9m: 37.4y [12.6–64.5, 20]; 9–12m: 33.0y [19.5–49.6, 19]; 12–24m: 32.4y [12.1–60.2, 44]; > 24m: 34.7y [12.4–62.4, 61]). VA endpoints at last visit were also similar regardless of T_i; however, minor differences were observed. Eyes that were 3–6m from symptom onset at treatment initiation had the best median [interquartile range (IQR)] VA at last visit (0–3m: 1.27 [0.40–1.68]; 3–6m: 1.22 [0.30–1.80]; 6–9m: 1.40 [1.02–1.56]; 9–12m: 1.52 [0.98–1.60]; 12–24m: 1.39 [0.58–1.74]; > 24m: 1.32 [0.90–1.62]) and the best median [IQR] VA change from last visit to baseline (0–3m: 0.08 [-0.20–0.62]; 3–6m: -0.20 [-0.46–0.10]; 6–9m: -0.11 [-0.19–0.00]; 9–12m: -0.10 [-0.48– -0.04]; 12–24m: -0.09 [-0.47–0.00]; > 24m: -0.06 [-0.18–0.00]).

Conclusions: LEROS study data provides important insights into the impact of time of treatment initiation on VA outcome. However, given the small number of eyes and large variation in the data, caution must be taken in interpreting these results.

References

1. Yu-Wai-Man P et al. Cell Rep Med. 2024;5:101437.

Aims/Purpose: Physicians in the UK can now request whole genome sequencing (WGS) for suspected inherited retinal disease (IRD), funded by a National Genomic Medicine Service. Achieving genetic diagnoses can be more challenging for ethnicities with lower representation in reference genomes. We report initial results from a specialist adult retinal service, with no prior experience of direct genetic testing from clinic.

Methods: WGS was undertaken with screening of a virtual panel of known IRD-associated genes. Exceptions to WGS included suspected albinism and pseudoxanthoma elasticum, where targeted sequencing was undertaken of albinism-associated genes or ABCC6 respectively.

Results: To date, results for 28 patients (mean (SD) age, 51 (17) years; 17 females) from 27 families have been received. Ethnicities were white European (13), African (9), South Asian (3), and mixed (3). Reports were positive in 15 cases (variants in ABCA4, BEST1, USH2A, TYR, COL18A1, RHO, ABCC6, PRPF8, CRB1, EFEMP1, NR2E3, CNGB3, CERKL) and were consistent with the clinical phenotype. Ten reports were negative and 3 were inconclusive. In 2 of the latter, the phenotype was sufficiently specific (Bietti crystalline dystrophy and Oguchi disease) to allow attribution of disease to variants found in CYP4V2 and GRK1 respectively. Of 17 patients achieving a genetic diagnosis, 9 were white European, 6 were of African origin, 1 South Asian, and 1 of mixed ethnicity). Mean (SD) time between patient consent and report availability was 9.0 (2.5) months.

Conclusions: In this analysis of tests performed so far from a clinic serving a multi-ethnic patient population, a molecular diagnosis was achieved in 61% of patients. The phenotype continues to be important for correctly interpreting genetic data. Achieving genetic diagnosis allows confirmation of genetic aetiology, more informed counselling relating to risk to children, and determines eligibility for the increasing number of gene-directed experimental therapies.

Aims/Purpose: To report anatomical and visual acuity outcome of surgical detachment of iridocorneal or lenticulocorneal adhesions in pediatric patients with Peters anomaly.

Methods: An observational study of pediatric patients with Peters anomaly treated with surgical detachment of iridocorneal or lenticulocorneal adhesions between 2011 and 2024 at the Helsinki University Hospital. Ocular and extraocular diagnoses, age, sex, best corrected visual acuity (BCVA), intraocular pressure, ocular surgeries, surgical complications, family history, genetics and anterior segment optical coherence tomography data were collected from the medical records.

Results: Fourteen eyes from nine patients underwent the surgery. The median age at the time of surgery was 14 months (range 5-56) and the median follow-up period was 156 months (range 6-166). Of the treated eyes, 12 had iridocorneal adhesions, while two eyes also had lenticulocorneal adhesions. No surgical complications were reported. Anterior segment anatomy and corneal clarity were stable or improved during the follow-up. Among the fourteen eyes, three eyes achieved a BCVA better than 0.3 (Snellen decimal), while none had a BCVA worse than 0.05. BCVA was not available for three treated eyes due to the patients' young age. The following additional ocular conditions were recorded: microphthalmia, cataract, glaucoma, retinal degeneration, nystagmus, corectopia and strabismus. Four eyes underwent further ocular surgeries, such as cataract surgery with intraocular lens implantation, anterior vitrectomy, pupilloplasty, and temporal iridectomy. Five patients had systemic findings. Pathogenic variants in the PITX3 and RARB genes were detected in three patients and one patient, respectively.

Conclusions: This study demonstrates that surgical detachment of irido- and even lenticulocorneal adhesions in Peters anomaly is safe and results stable anatomical and visual acuity outcome over the period of critical visual development.

Aims/Purpose: This work aims to assess the consistency of the neurovascular coupling (NVC) response of a healthy subject group at two visits 5 months apart.

Methods: Fundus images (4x4 degrees) of the macular region (3 baseline acquisitions and 3 acquisitions each preceded by flicker stimulation) of 13 healthy adults (one eye each, age (m+/-sd): 33.08+/-11.4 yrs.) were acquired with the rtx1 AO camera (ImagineEyes, Orsay, France). The rtx1 was configured for a 20 second, 15Hz, and 4 μW.cm^(-2) flicker stimulation over a 29x20 degrees area of the imaged eye fundus. Acquisitions for each subject were repeated 159+/-17 (m+/-sd) days later.

The lumen of retinal arterioles within the macular region was determined using custom-developed software for the automatic segmentation of lumen-wall interfaces. An experienced technician visually assessed all segmentations to reject subpar results. Lumen diameters were calculated from the distance between the lumen-wall interfaces as measured perpendicular to the vessel axis along a vessel segment. NVC responses were calculated per eye as the relative difference (%) of the lumen diameter after stimulation to the respective baseline.

Results: A total of 22 out of 156 images (14%) were rejected and excluded from further analysis. NVC responses ranged from 0.21% to 8.28% (3.36+/-2.75 % (m+/-sd)) for the first visit and from -0.24% to 9.13% (3.03+/-2.54 %) for the follow-up. Response differences between time points ranged from -6.15% (8.28% to 2.13%) to 4.21% (0.98% to 5.19%), with an average (sd) of 0.34(3.03) % for the group. A paired sample Wilcoxon test between time points is not statistically significant (p = 0.650), supporting that the responses are consistent between time points.

Conclusions: The obtained results show a consistent NVC response for a 5-month interval.

Clinical signs of acanthamoeba keratitis are in early stages grey-dirty epithelium, pseudodendritiformic epitheliopathy, perineuritis, multifocal stromal infiltrates, ring infiltrate and in later stages scleritis, iris atrophy, anterior synechiae, secondary glaucoma, mature cataract, and chorioretinitis. Beside triple-topical-therapy, in therapy resistant cases surgical treatment options such as corneal cryotherapy, amniotic membrane transplantation, riboflavin-UVA crosslinking, and penetrating keratoplasty are applied.